Dapsone is an effective therapy for the skin lesions of subacute cutaneous lupus erythematosus and urticarial vasculitis in a patient with C2 deficiency.
The deficiency of second component of complement (C2d) is the most common hereditary complement deficiency. Patients with C2 deficiency are frequently associated with an auto-immune disease process, in particular, systemic lupus erythematosus (LE)-like syndrome and/or vasculitic syndrome or bacterial infections. C2d has been associated with the LE subset of subacute cutaneous LE (SCLE), the presence of anti-Ro (SSA) antibodies, and the human leukocyte antigen (HLA) types A10, B18, DR2. We describe the clinical, serologic and immunogenetic data in a patient with manifestations of Sjögren's syndrome who developed urticarial vasculitis and photosensitive annular SCLE which were effectively treated with oral dapsone. Our case illustrates the dynamic nature of LE.
Holtman
J H
JH
School of Medicine, University of Louisville, KY.
Neustadt
D H
DH
Klein
J
J
Callen
J P
JP
D000284:Administration, Oral; D000328:Adult; D003175:Complement C2 / Q000172:deficiency*; D003622:Dapsone / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008178:Lupus Erythematosus, Cutaneous / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications / Q000378:metabolism / Q000473:pathology; D014581:Urticaria / Q000150:complications; D014657:Vasculitis / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology
D003175:Complement C2; D003622:Dapsone